Pediatric nephrology : journal of the International Pediatric Nephrology Association
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Renal anomalies are common in patients with Bardet-Biedl syndrome (BBS), a renal cystic ciliopathy with multi-systemic features. Renal transplantation is indicated in cases of end-stage renal disease (ESRD), but transplant centers may be hesitant to perform the necessary transplant in light of the multitude of metabolic comorbidities these patients often face with the potential to complicate outcomes. ⋯ At a median follow-up time of 97 months, relatively few complications of renal transplantation were reported in the patients of this study. However, body mass index was significantly elevated in transplanted individuals compared to non-transplanted individuals participating in CRIBBS at the most recent follow-up. Although the frequency of obesity and other manifestations of the metabolic syndrome warrant meticulous management in this high-risk population, favorable long-term outcomes suggest that renal transplantation is a viable option for patients with BBS and ESRD.
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Continuous renal replacement therapy (CRRT) has emerged as the modality of choice for the management of high-risk neonates with acute kidney injury (AKI), inborn errors of metabolism and multi-organ dysfunction. The aim of this study was to evaluate fluid overload (FO) and investigate the factors associated with outcomes in neonates undergoing CRRT. ⋯ Neonates with a higher percentage FO and higher levels of serum creatinine at CRRT initiation showed poor outcomes. Early initiation of CRRT before the development of severe FO or azotemia might improve the outcomes of neonates requiring CRRT.
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Editorial Comment
Neonatal extracorporeal renal replacement therapy-a routine renal support modality?
Peritoneal dialysis (PD) is generally considered the preferred extracorporeal therapy for neonates with acute kidney injury (AKI). However, there are situations when PD is not suitable, such as in patients with previous abdominal surgery, hyperammonemia and significant ascites or anasarca. Additionally, with a need to start PD soon after catheter placement, there is increased risk of PD catheter leak and infection. ⋯ In addition, ultrafiltration often cannot be set in sufficiently small increments to be suitable for neonates. Although CRRT practices can be modified to fit the needs of infants and neonates, there is a need for a device designed specifically for this population. Until that becomes available, providing the highest level of care for neonates with AKI is dependent on the shared experiences of members of the pediatric nephrology community.
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To describe the safety and efficacy of sodium polystyrene sulfonate (SPS) in pediatric patients with acute hyperkalemia. ⋯ SPS was used effectively and safely in the majority of patients in this report. However, it may not be appropriate as a first single-line agent in patients with severe acute hyperkalemia who require a greater than 25 % reduction in serum potassium levels or those at a high risk for cardiac arrhythmias.